The Strange Increase in Prion Diseases
Hopeful interpretation: better diagnosis. Scary interpretation: a new exposure
In 1986, in Britain, cattle started dying.
The condition, quickly nicknamed “mad cow disease” was clearly infectious, but the particular pathogen was difficult to identify. By 1993, 120,000 cattle in Britain were identified as being infected. As yet, no human cases had occurred and the UK government insisted that cattle were a dead end host for the pathogen. By the mid-90’s, however, multiple human cases — attributable to ingestion of meat and organs from infected cattle, were discovered. In humans, variant Creutzfeldt-Jakob Disease was a media sensation — a nearly uniformly fatal, untreatable condition with a rapid onset of dementia, mobility issues characterized by jerky movements, and autopsy reports finding the brain itself had been turned into a spongy mess.
The US banned UK beef imports in 1996 and only lifted the ban in 2020.
The disease was made all the more mysterious because the pathogen involved was not a bacteria, parasite, or virus, but a protein — or a proteinaceous infectious particle — shortened to “prion”.
Prions are misfolded proteins that aggregate in cells — in this case in nerve cells. But what makes prions different than other…
